Myelofibrosis and idiopathic thrombocytopenic purpura

Seelen, Marc A.; Meijer, Paul H. E.; Posthuma, E. F. M.; Meinders, A. E.

doi:10.1007/s002770050327

Cited by 7 publications

(3 citation statements)

References 13 publications

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“…The frequent association of IM with immunological phenomena has been taken as an argument supporting a possible immune basis to the disease. IM-associated immunological features include the presence of circulating immune complexes in a high proportion of patients (4±6, 19), as well as anti-nuclear and other anti-tissue autoantibodies (6, 7), lupus-type circulating anticoagulants (6), anti-platelet antibodies (7,12) and a positive direct Coombs' test (6,7). Besides these, lymphoid nodules in the bone marrow can be observed in a quarter of IM patients (5,8).…”

Section: Discussionmentioning

confidence: 99%

“…A possible immune basis of the disease has been postulated based on the presence of immunologic features in a proportion of patients, including circulating immune complexes, positive autoimmune tests and bone marrow lymphoid nodules (4±8). The occasional association of IM with autoimmune disorders, such as scleroderma (9), systemic lupus erythematosus (10,11), idiopathic thrombocytopenic purpura (7,12), and Graves' disease (13) would give support to the above hypothesis. It is not known, however, whether the latter associations are merely coincidental and whether the above immunologic ®ndings have a role in the pathogenesis of IM.…”

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confidence: 93%

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Assessment of peripheral blood lymphocyte subsets in idiopathic myelofibrosis

Cervantes

Hernández‐Boluda

Villamor

et al. 2000

European J of Haematology

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The objective of this study was to contribute to a better characterization of the immunological profile of idiopathic myelofibrosis (IM) at presentation by analysing the blood lymphocyte subsets and their possible correlations with other disease features. Absolute blood lymphocytes and lymphocyte subsets were assessed in 31 IM patients, compared with those from 34 healthy individuals, and correlated with the patients' main clinical, hematological and bone marrow histologic features. The mean lymphocyte count of the IM patients was 1.1 (SD 0.6) x 10(9)/L, versus 1.6 (SD 0.49) x 10(9)/L in controls (p = 0.0006), with 24 of the 31 patients (77.4%) showing lymphocytopenia (< 1.5 x 10(9)/L). IM patients had significantly lower counts of CD3, CD4, CD8, and CD3 -/ CD56+ cells, and significantly higher CD3 +/CD56 + lymphocyte counts. Although no significant differences were found between patients and controls with regard to CD19+/CD5+ cell counts, increased CD5 + B-cell lymphocytes were observed in three IM patients. In one of the latter patients, Ig gene rearrangement analysis of the heavy chain gene demonstrated such a subpopulation to be clonal, but the patient did not develop features of chronic lymphoid leukemia during a 5-yr follow-up. No correlation was found between the patients' blood lymphocyte counts and other disease features. We conclude that most IM patients have absolute lymphopenia, decreased T cells and increased cytotoxic T cells at diagnosis, and 10% of them show an increased CD5 + B-cell subpopulation.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 93%

Assessment of peripheral blood lymphocyte subsets in idiopathic myelofibrosis

Cervantes

Hernández‐Boluda

Villamor

et al. 2000

European J of Haematology

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“…Breath test for H. pylori infection was negative in all patients. Given that the causative role of this pathogenic bacteria in the setting of thrombocytopenia has been demonstrated only in recent years,[8–10] its presence was tested in two patients during the initial work-up and in the remaining case several years after the onset of the hematological disorders. The morphological examination of peripheral blood showed marked thrombocytopenia and platelet anisocytosis but not other significant abnormalities.…”

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confidence: 99%

Idiopathic thrombocytopenic purpura coexisting with polycythemia vera

Niscola

Giovannini

Scaramucci

et al. 2011

Asian J Transfus Sci

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Idiopathic Myelofibrosis Associated with Classic Polyarteritis Nodosa

Camós

Arellano‐Rodrigo

Abelló

et al. 2003

Leukemia & Lymphoma

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A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.

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Myelofibrosis and idiopathic thrombocytopenic purpura

Abstract: A case of idiopathic myelofibrosis (IMF) with concomitant autoimmune thrombocytopenic purpura (AITP) is reported. The literature on platelet antibodies in IMF is reviewed.

Cited by 7 publications

References 13 publications

Assessment of peripheral blood lymphocyte subsets in idiopathic myelofibrosis

Assessment of peripheral blood lymphocyte subsets in idiopathic myelofibrosis

Idiopathic thrombocytopenic purpura coexisting with polycythemia vera

Idiopathic Myelofibrosis Associated with Classic Polyarteritis Nodosa

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